Reduced muscle mitochondrial enzyme activity in MuSK-immunized mice

Özkök, Elif; Durmuş, Hacer; Yetimler, Berrak; Tașlı, Hatice; Τράκας, Νικόλαος; Ulusoy, Canan; Lagoumintzis, George; Tzartos, Socrates J.; Tüzün, Erdem

doi:10.5414/np300875

Cited by 8 publications

(2 citation statements)

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“…The association between MuSK Abs and myopathy is still an unsolved question. Muscle biopsy studies in MuSK + MG patients reported myopathic features and mitochondrial abnormalities, the latter having been more recently described in MuSK‐immunized animals . These findings open interesting perspectives and deserve further investigation.…”

Section: Clinical Aspectssupporting

confidence: 53%

Myasthenia gravis with antibodies to MuSK: an update

Evoli

Alboini

Damato

et al. 2017

Annals of the New York Academy of Sciences

113

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Myasthenia gravis with antibodies to the muscle-specific tyrosine kinase (MuSK MG) is a rare disease with distinctive pathogenic mechanisms and clinical features. An acute onset and predominant bulbar muscle weakness are very common and highly suggestive of the disease. On the other hand, a more indolent course, atypical ocular presentation, and signs of cholinergic hyperactivity may complicate the diagnosis. Though MuSK MG is still a severe disease, over the years we have observed a steady reduction in the rate of respiratory crisis and a significant improvement in the clinical outcome, both likely related to earlier diagnosis and timely treatment. Despite the improved management, MuSK MG patients tend to remain dependent on long-term immunosuppressive treatment and may develop permanent disabling weakness. In uncontrolled studies, B cell depletion with rituximab proved effective in most patients with refractory disease, inducing prolonged clinical responses associated with a sustained reduction of serum antibody levels. Promising results from experimental studies and case reports suggest that both 3,4-diaminopyridine and albuterol may be effective as symptomatic agents.

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Section: Clinical Aspectssupporting

confidence: 53%

Myasthenia gravis with antibodies to MuSK: an update

Evoli

Alboini

Damato

et al. 2017

Annals of the New York Academy of Sciences

113

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“…Although the immune mechanisms underlying neuromuscular diseases, such as MG, has been extensively investigated, there are relatively few studies on muscle cell damage and mitochondrial dysfunction. MG autoantibodies do not only induce AChR destruction directly, but also cause intracellular mitochondrial changes ( 32 ). In a previous experimental autoimmune MG (EAMG) rat study, it was observed that both mitochondrial structure and skeletal muscle function were compromised to varying degrees ( 15 ).…”

Section: Mitochondrial Dysfunction Disordersmentioning

confidence: 99%

The mitochondrial biogenesis signaling pathway is a potential therapeutic target for myasthenia gravis via energy metabolism (Review)

Song

et al. 2021

Exp Ther Med

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Myasthenia gravis (MG) is an autoantibody-mediated autoimmune disease that is characterized by muscle weakness and fatigue. Traditional treatments for MG target the neuromuscular junction (NMJ) or the immune system. However, the efficacy of such treatments is limited, and novel therapeutic options for MG are urgently required. In the current review, a new therapeutic strategy is proposed based on the mitochondrial biogenesis and energy metabolism pathway, as stimulating mitochondrial biogenesis and the energy metabolism might alleviate myasthenia gravis. A number of cellular sensors of the energy metabolism were investigated, including AMP-activated protein kinase (AMPK) and sirtuin 1 (SIRT1). AMPK and SIRT1 are sensors that regulate cellular energy homeostasis and maintain energy metabolism by balancing anabolism and catabolism. Peroxisome proliferator-activated receptor γ coactivator 1α and its downstream transcription factors nuclear respiratory factors 1, nuclear respiratory factors 2, and transcription factor A are key sensors of mitochondrial biogenesis, which can restore mitochondrial DNA and produce new mitochondria. These processes help to control muscle contraction and relieve the symptoms of MG, including muscle weakness caused by dysfunctional NMJ transmission. Therefore, the present review provides evidence for the therapeutic potential of targeting mitochondrial biogenesis for the treatment of MG.

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